Bemba M, Guardiola P, Garderet L, Devergie A, Ribaud P, Esperou H, Noguera M H, Gluckman E, Socié G
Service d'Hématologie-Greffe de Moelle, Hôpital Saint-Louis, Paris Cedex, France.
Br J Haematol. 1999 May;105(2):366-8.
Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal disorder of the haemopoietic stem cells for which the only curative treatment is bone marrow transplantation. There are few reports on the use of allogeneic transplantation for PNH, and nearly all of them include only a few patients. Between September 1978 and December 1997, 16 patients underwent marrow transplantation for PNH at the Hospital Saint Louis. The 5-year survival rate for the 16 patients was 58 +/- 13%. Two factors, an absolute neutrophil count >1.0 x 109/l and haemoglobin level >9 g/dl at transplant, were found to be statistically associated with a better outcome.
阵发性夜间血红蛋白尿(PNH)是一种造血干细胞的获得性克隆性疾病,唯一的治愈性治疗方法是骨髓移植。关于异基因移植治疗PNH的报道很少,几乎所有报道都只涉及少数患者。1978年9月至1997年12月期间,16例阵发性夜间血红蛋白尿患者在圣路易医院接受了骨髓移植。这16例患者的5年生存率为58±13%。研究发现,移植时两个因素与较好的预后在统计学上相关,即绝对中性粒细胞计数>1.0×10⁹/L和血红蛋白水平>9g/dl。